Progressive myelin oligodendrocyte glycoprotein-associated demyelination mimicking leukodystrophy

Abstract

Background:

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) may be associated with relapsing disease, but clinical progression independent of relapse activity is rare.

Objectives:

To report progressive disease in a patient with MOGAD.

Methods:

A single retrospective case report.

Results:

At 4 years of age, the patient had a single episode of acute disseminated encephalomyelitis. She remained well until age 17 years but over the next 9 years developed progressive spastic quadriparesis, cognitive and bulbar dysfunction. Brain imaging showed a leukodystrophy-like pattern of white matter abnormality with contrast enhancement at different time points. Myelin oligodendrocyte glycoprotein (MOG)-IgG was repeatedly positive by live cell-based assay.

Conclusion:

Secondary progression may be a rare presentation of MOG-IgG-associated disease.